Treatment Of Idiopathic Retroperitoneal Fibrosis Research Articles

SIGNIFICANCE OF IgG4 IN IDIOPATHIC RETROPERITONEAL FIBROSIS

(Objective)Retroperitoneal fibrosis is largely divided into the idiopathic and secondary types. Some idiopathic cases include IgG4-related diseases, which are often similar to malignant diseases, such as lymphoma and sarcoma. The diagnostic criteria for IgG4-related disease are used and pathologic examination is necessary for a definitive diagnosis of IgG4-related retroperitoneal fibrosis. The first choice of treatment for IgG4-related retroperitoneal fibrosis is steroid administration, but no consensus has been established regarding its dose and tapering schedule. We investigated the significance of IgG4 in diagnosis and treatment of idiopathic retroperitoneal fibrosis. (Patients and methods)We examined 14 cases diagnosed as idiopathic retroperitoneal fibrosis between April 2013 and March 2019. Serum IgG4 was measured at the time of diagnosis in 13 cases, and changes over time in serum IgG4 before and after the induction of steroid therapy were measured in 6 cases. Computed tomography-guided biopsy was performed on 4 cases. (Results)Of all cases, 1 patient was diagnosed as IgG4-related retroperitoneal fibrosis and 5 patients were classified as possible group. Ten patients were administered steroid therapy. Percutaneous nephrostomy tube was placed in 3 patients and was removed in 2 of these patients after steroid therapy. The serum high levels of IgG4 were confirmed in all 4 patients who were classified into the possible group and who were treated with steroids. (Conclusion)Although histologic examination is necessary for the diagnosis of retroperitoneal fibrosis, tissue collection by open or laparoscopic surgery is highly invasive. CT-guided biopsy may be useful in high-risk cases, such as elderly patients on anticoagulation. After excluding other diseases in high-risk cases, response to empiric steroid therapy may be diagnostic. In the possible group, changes in serum IgG4 levels may reflect the disease condition and might be useful in determining the maintenance dose of steroids.

Successful treatment of idiopathic retroperitoneal fibrosis with combined immunosuppressive therapy

Background/Aim. Idiopathic retroperitoneal fibrosis (IRF) is characterized by the fibroinflammatory periaortic tissue that affects the ureters, causing obstructive nephropathy and variable impairment of renal function. The findings strongly suggest an autoimmune etiology. The optimal treatment has not been established. The aim of this study was to analyze a long-term efficacy of combined corticosteroid therapy with mycophenolate mofetil (MMF) in the patients with IRF. Methods. We retrospectively followed 13 patients (8 males and 5 females) with IRF. All patients received corticosteroids and MMF. For the patients with severe renal failure, an initial ureteral decompression was made and prednisone was started orally 0.5 mg/kg with fast tapering. In cases with a mild renal failure corticosteroids were administrated as intravenous methylprednisolone pulses for 3 days, followed by oral prednisone. The dose of MMF was 1000 mg twice a day. MMF was stopped after 18 months and prednisone after 48 months. Results. Systemic symptoms resolved in all patients. Erythrocyte sedimentation (SE) rate declined from the mean of 67.6 to 26.3 mm/h and C-reactive protein (CRP) from the mean of 18.5 to 6.3 mg/L. In 7 out of 8 patients, the ureteral stents were successfully removed 13 weeks on average. Seven patients had 100% of reduction in the periaortic mass, and the average percent reduction was 76.9%. The kidney function improved and remained normal in 6 treated patients. In 4 patients a mild chronic renal failure remained due to afunction of one kidney. Three patients, with a prior chronic renal failure, did not get worse renal function. The disease recurred in 3 patients. There were no treatment side effects noted. Conclusion. Combination of corticosteroids and MMF is a potentially effective treatment in restoring the renal function and reducing the fibrotic tissue in the patients with idiopathic retroperitoneal fibrosis. It could prevent the need for ureteral stenting and surgery. Longer treatment may reduce a possibility of recurrence.

AB0816 Efficacy of biological agents in the treatment of idiopathic retroperitoneal fibrosis

ObjectivesGlucocorticoids (GCs) are the most used drugs for the treatment of idiopathic retroperitoneal fibrosis (IRF).Several steroid-sparing agents, including immunodepressants and tamoxifen, have been used with varying success. However, some patients...

Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial

Glucocorticoids are the mainstay of treatment of idiopathic retroperitoneal fibrosis, but they often have substantial toxic effects. Several reports have suggested tamoxifen as an alternative to glucocorticoids. We compared the efficacy of prednisone with that of tamoxifen in maintainance of remission in patients with idiopathic retroperitoneal fibrosis. In this open-label, randomised controlled trial, we enrolled patients aged 18-85 years with newly diagnosed idiopathic retroperitoneal fibrosis at the Parma Hospital, Parma, Italy, between Oct 1, 2000, and June 30, 2006. After induction therapy with 1 mg/kg daily of prednisone for 1 month, the patients who achieved remission were randomly assigned to receive tapering prednisone (initial dose 0·5 mg/kg daily) for 8 months or tamoxifen (fixed dose 0·5 mg/kg daily) for 8 months. The sequence of randomisation (1:1), blocked in groups of two and four (with block size randomly selected), was generated by the trial statistician with a computer programme. After the end of treatment, the patients were followed up for an additional 18 months. Neither patients nor those giving interventions or analysing the data were masked to group assignment. The two radiologists who assessed CT and MRI scans were masked. The primary endpoint was the relapse rate by the end of treatment (month 8), which was analysed by intention to treat. This trial is registered with ClinicalTrials.gov, number NCT00440349. After induction therapy, 36 of the 40 enrolled patients achieved remission and were randomly assigned to treatment (18 per group). One patient (6%) in the prednisone group and seven patients (39%) in the tamoxifen group relapsed by the end of treatment (difference -33% [95% CI -58 to -8, p=0·0408]. The difference in relapse rate between the groups was sustained after the additional 18-month follow-up: the 26-month estimated cumulative relapse probability was 17% with prednisone and 50% with tamoxifen (difference -33% [-62 to -3, p=0·0372]). Cushingoid changes and grade 2 hypercholesterolaemia were more common in the prednisone group than in the tamoxifen group (p=0·0116 and p=0·0408, respectively). Prednisone is more effective in prevention of relapses than is tamoxifen in patients with idiopathic retroperitoneal fibrosis. Therefore, prednisone should be considered as first-line treatment for patients with newly diagnosed idiopathic retroperitoneal fibrosis. Parma University Hospital.

Mycophenolate mofetil combined with corticosteroids in treatment of idiopathic retroperitoneal fibrosis:a case report

Mycophenolate mofetil combined with corticosteroids in treatment of idiopathic retroperitoneal fibrosis:a case report

Treatment of Idiopathic Retroperitoneal Fibrosis With Colchicine and Steroids: A Case Series

Idiopathic retroperitoneal fibrosis (IRPF) is an uncommon, but increasingly recognized, disease. Autoimmune mechanisms probably participate in its pathogenesis. Patients have been treated with high-dose steroids and sometimes immunosuppressive drugs, resulting in toxicity risk during prolonged treatments; thus, it would be wise to look for safer drugs. Colchicine has antifibrotic, anti-inflammatory, and immunosuppressive effects. Hence, it is a potential therapeutic resource. Case series. 7 consecutives patients with IRPF diagnosed since 1993 at a navy hospital were prescribed colchicine plus prednisone or deflazacort. After reaching a clinical response, the steroid dose was tapered, maintaining daily colchicine therapy. Symptomatic improvement was defined as time to relief of symptoms; clinical improvement, time to normalization of inflammatory parameters with symptomatic improvement; radiological changes, percentage of decrease in retroperitoneal mass; recurrence, as the need to increase steroid dose or add immunosuppressors because of reactivation of inflammation; and treatment failure, as persistence of signs of active inflammation and/or decrease in retroperitoneal mass less than 50% and/or deterioration in kidney function at the last visit. At month 3 of treatment, all patients had symptomatic improvement and 67% had clinical improvement. Between 8 and 12 months, 67% of patients had a decrease in retroperitoneal mass greater than 50%. No recurrence or treatment failure was observed during follow-up (mean, 72.5 months). At the last visit, all patients had a significant decrease in retroperitoneal mass (mean, 79.8% +/- 19.7%). One patient died of septic shock on day 21 of treatment. This series did not have a control group. Colchicine in association with a low induction dose of steroids (<0.5 mg/kg/d) produced remission rates in patients with IRPF similar to those for regimens using greater steroid doses alone or in combination with immunosuppressive drugs. In addition, colchicine may have contributed to recurrence prevention.

Chronic Periaortitis

Chronic Periaortitis

Laparoscopic Ureterolysis and Omental Wrapping

To describe our laparoscopic technique of ureterolysis and omental wrapping using the LigaSure device for the treatment of idiopathic retroperitoneal fibrosis. Four bilateral laparoscopic ureterolyses (LUs) and two unilateral LUs were performed in 6 male patients (mean age 47 years). Of the 6 patients, 4 underwent LU without having undergone medical therapy before surgery and 2 underwent LU after medical therapy failure. All had had ureteral stents placed before surgery. The ureters were completely freed from the fibrotic tissue using an Overholt laparoscopic forceps and 10-mm LigaSure atlas. An omental wrap was passed behind the colonic flexure, placed around the ureter, and fixed to the psoas muscle. The mean operating time was 80 minutes (range 75 and 85) for the unilateral LUs and 200 minutes (range 180-225) for the bilateral procedures. The mean blood loss was 75 mL (range 50 and 100) during LUs and 150 mL (range 80-220) during bilateral LUs. The mean hospital stay was 3.33 days (range 2-5). All indwelling ureteral stents were removed at 4 weeks postoperatively. At a mean follow-up of 37.5 months (range 23-59), all patients were free of symptom and all renal units were unobstructed. In our experience of LUs and omental wrapping, the reproduction of each step of open surgery seems to offer excellent midterm outcomes. The use of the LigaSure simplified the laparoscopic procedure and made it feasible and safe. We believe that the minimally invasive nature and high effectiveness of LU suggest consideration of this procedure as first-line treatment of idiopathic retroperitoneal fibrosis.

Long-Term Renal and Patient Outcome in Idiopathic Retroperitoneal Fibrosis Treated With Prednisone

Primary medical treatment of idiopathic retroperitoneal fibrosis (RPF) increasingly is accepted. However, the optimum treatment strategy is still unclear. Case series. Single tertiary care referral center. 24 patients with idiopathic RPF treated with prednisone for 1 year, if needed, with (urgent) renal drainage from June 1991 through October 2006. Clinical improvement, laboratory parameters, repeated computed tomographic (CT) scanning. Treatment was considered successful if the following criteria were met at the end of the 1-year treatment period: significant subjective clinical improvement, (near-)normalization of acute-phase reactants, improvement in renal function with disappearance of ureteral obstruction, and CT-documented mass regression. Recurrence is defined as need for retreatment because of return of signs and symptoms after the 1-year treatment period in patients with initial treatment success. 22 patients reported significant to complete resolution of symptoms after median treatment duration of 2.0 weeks (0.7 to 3.0). Follow-up showed decreases in erythrocyte sedimentation rate, C-reactive protein level (both P < 0.0001), and serum creatinine level (P = 0.0230) at 6 weeks, which persisted during the treatment period. Repeated CT scanning showed mass regression in 19 patients during the treatment period. Six patients were considered treatment failures, and there were 23 recurrences 10 months (7 to 14) after prednisone withdrawal in 13 of 18 patients with initial treatment success. At the end of follow-up (median, 55 months), 7 patients had impaired renal function; 1 patient reached end-stage renal disease. The mortality rate was 8%. There was no comparison with other treatments. One-year treatment with prednisone is associated with a high rate of initial success, but a high recurrence rate. Despite frequent disease relapse, long-term renal and patient outcome was good.

Mycophenolate mofetil combined with steroids: New experiences in the treatment of idiopathic retroperitoneal fibrosis

[corrected] Idiopathic retroperitoneal fibrosis (IRF) is an uncommon disease characterized by a retroperitoneal fibrotic tissue that often involve the ureters, leading to the obstructive nephropathy and variable impairment of renal function. Findings strongly suggest an autoimmune etiology. Surgery, medical treatment with immunosuppressive drugs, or a combination of both are proposed. The optimal treatment has not been established yet. The aim of this study was to present our experience with combined immunosuppressive therapy of IRF, steroids (S) and mycophenolate mofetil (MMF). We prospectively followed four patients with IRF from January 2004 to December 2006. Three patients had an active disease with bilateral hydronephrosis. In the two of them acute renal failure was presented, and ureteral catheters were inserted in one in order to manage ureteral obstruction. One patient has came to our unit with a relapse of IRF and incipient chronic renal failure after the prior therapy with ureterolysis and immunosuppressive drugs (azathioprine and tamoxifen). All patients received steroids and MMF. Two patients were treated with intravenous methylprednisolone pulses (250 mg each), for three consecutive days, followed by oral prednisone 0.5 mg/kg/day. The other two patients received oral prednisone at the same dose. Prednisone was gradually tappered to a maintenance dose of 10 mg/kg/day. Simultaneously, all patients received MMF, initially 1 g/day with the increase to 2 g/day. After four weeks of the therapy all symptoms disappeared, as well as a hydronephrosis with a decrease of erythrocyte sedimentation rate and Creactive protein (CRP) to normal level in all patients. Three patents remain in remission untill the end of the follow up. One patient had a relapse because of stopping taking the therapy after six months. He was treated by oral prednisone 0.5 mg/kg/day, which was gradually decreased. After twelve weeks hydronephrosis disappeared and CRP returns to the normal level. The combination of steroids and mycophenolate mofetil led to the remission of IRF with a strong and quick immunosuppressive effect. It also provided avoiding the long-term use of high steroid dose and surgical procedures.

Surgical treatment of idiopathic retroperitoneal fibrosis by ureterolysis and ureteral protection with a posterior preperitoneal fat flap

Surgical treatment of idiopathic retroperitoneal fibrosis by ureterolysis and ureteral protection with a posterior preperitoneal fat flap

La terapia chirurgica tradizionale della fibrosi retroperitoneale idiopatica: Traditional surgical treatment of idiopathic retroperitoneal fibrosis

Surgical treatment of idiopathic retroperitoneal fibrosis is indicated in the most advanced stages of the disease and aims at resolving the urinary obstruction by freeing the ureters of the compressing and enveloping fibrous plaque. The purpose of this study was to briefly review the traditional surgical techniques, while assessing the results of the main reports in literature.

Successful Treatment of Idiopathic Retroperitoneal Fibrosis with Steroids

Idiopathic retroperitoneal fibrosis (IRF) may cause ureteric obstruction with renal damage. Ureterolysis with intraperitonealization of the ureter is commonly used as primary treatment. We gave corticosteroids for two years to six patients, four males and two females, between 40 and 56 years of age. one of the patients is in his tenth month of treatment and another in the fourth month. Between 4 and 74 months (mean 40.4 months) after initiation of treatment, kidney function had improved, or was preserved in previously functioning renal units, and the patients were free from symptoms. We conclude that steroids may be used as primary treatment of IRF after histological or cytological diagnosis to exclude retroperitoneal malignancy. Steroids should also be part of the treatment strategy in patients with a more aggressive disease who may need surgery. Patients with IRF should be followed for the rest of their lives after discontinuation of steroid therapy.

Idiopathic retroperitoneal fibrosis.

Herein is described a case report of urinary tract obstruction due to idiopathic retroperitoneal fibrosis (IRF) in a 43-year-old man. Recent studies suggest that IRF fits into the broader pathologic heading of chronic periaortitis. Although there may be more than one pathophysiologic mechanism, growing evidence suggests that many cases of IRF are due to an autoallergic reaction to lipid material in the atheromatous aorta. Computed tomographic scanning has supplanted excretory and retrograde urography in the diagnosis of this condition. Ureterolysis and steroids remain the mainstays in the treatment of IRF. Even after successful decompression of urinary obstruction, long-term follow-up is required in these patients because of the possibility of other complications such as vena caval or duodenal obstruction.

Treatment of idiopathic retroperitoneal fibrosis by immunosuppression : W.S. McDougal and R.C. MacDonell, Jr. J Urol 145:112–114, (January), 1991

Treatment of idiopathic retroperitoneal fibrosis by immunosuppression : W.S. McDougal and R.C. MacDonell, Jr. J Urol 145:112–114, (January), 1991

Treatment of Idiopathic Retroperitoneal Fibrosis by Immunosuppression

AbstractIdiopathic retroperitoneal fibrosis is exceedingly uncommon in childhood and its etiology is uncertain. Support for an immunological basis for the disease is given by a report of a 14-year-old girl with severe retroperitoneal fibrosis causing progressive azotemia in whom azathioprineand prednisolone were used successfully. This case supports the efficacy of immunotherapy in the treatment of idiopathic retroperitoneal fibrosis